RNU2-2

Chr 11AD

RNA, U2 small nuclear 2

Also known as: DEE119, RNU2-2P, RNU2B, U2

This gene encodes the U2 small nuclear RNA, which is an essential component of the spliceosome that removes introns from pre-mRNA transcripts during RNA processing. Mutations cause developmental and epileptic encephalopathy 119, characterized by early-onset seizures and developmental delays affecting the central nervous system. The condition follows an autosomal dominant inheritance pattern.

OMIMResearchSummary from OMIM
AD1 OMIM phenotype
Clinical SummaryRNU2-2
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Gene-Disease Validity (ClinGen)
developmental and epileptic encephalopathy 119 · ADStrong

Strong evidence — appropriate for clinical testing

Some data sources returned errors (1)

gnomad: Error: Gene not found

Population Genetics & Constraint

Constraint data not available from gnomAD.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

RNU2-2 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

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